Abstract:We conducted an international study to evaluate practices in the diagnosis and management of patients with chronic thromboembolic pulmonary hypertension (CTEPH) globally across different regions. Between August and October 2012, CTEPH-treating physicians completed a 15-minute online questionnaire and provided patient record data for their 2-5 most recent patients with CTEPH. Overall, 496 physicians (Europe: 260; United States: 152; Argentina: 52; Japan: 32) completed the questionnaire and provided patient record data for 1,748 patients. The proportion of physicians who described themselves as working in or affiliated with a specialized pulmonary hypertension (PH) center ranged from 38% in France and Italy to 83% in the United States. A large proportion of patients did not undergo ventilation/perfusion scanning (46%-67%) or right heart catheterization (24%-57%) for the diagnosis of CTEPH. Referral rates for pulmonary endarterectomy evaluation ranged from 25% in Japan to 44% in Europe, with higher referral rates in PH centers; the main reasons for lack of referral were that surgery was not considered unless medical treatment was failing and patient refusal. Other variations in management included greater use of phosphodiesterase 5 inhibitors in the United States than in Europe and Japan and greater use of combination treatment in the United States than in Europe. Physicians' perceptions of their treatment strategy were generally consistent with patient record data. Results from this study, which includes a global aspect of CTEPH care, demonstrate not only regional differences in CTEPH management but, more importantly, considerable nonadherence to the diagnosis and treatment guidelines for CTEPH, even in PH centers.Keywords: chronic thromboembolic pulmonary hypertension, pulmonary arterial hypertension-specific therapies, diagnosis, clinical practice, guidelines. Chronic thromboembolic pulmonary hypertension (CTEPH) is a disease of obstructive pulmonary artery remodeling as a consequence of major vessel thromboembolism. 1 A ventilation/perfusion (V/Q) scan is recommended in the workup of all patients with pulmonary hypertension (PH) to screen for CTEPH. 1 Diagnosis of CTEPH is challenging for several reasons, including nonspecific presenting symptoms, which may occur late in the progression of the disease, and a lack of prior pulmonary embolism in a high percentage of patients. 2 Once CTEPH is diagnosed, pulmonary endarterectomy (PEA) is the gold-standard treatment for these patients and is potentially curative. 1,3 However, between 20% and 40% of these patients are considered to have inoperable CTEPH, 4 and approximately 30% may have residual PH after PEA (defined as mean pulmonary artery pressure of ≥30 mmHg at 3-month follow-up after surgery). 5 Balloon pulmonary angioplasty (BPA) is an emerging treatment option for patients with inoperable or persistent CTEPH. 3 However, the role of BPA in CTEPH has not been investigated in randomized trials, and further studies are warranted; current guidelines state ...

Abstract:We conducted an international study to evaluate practices in the diagnosis and management of pulmonary arterial hypertension (PAH) globally across different geographic regions. Between July and October 2012, PAH-treating physicians completed a 15-minute online questionnaire and provided patient record data for their 3 or 5 most recent patients with PAH. Overall, 560 physicians (Europe: 278; United States: 160; Argentina: 53; Japan: 69) completed the questionnaire and provided data for 2,618 patients. The proportion of physicians who described themselves as working in or affiliated with a specialized pulmonary hypertension center ranged from 13% in Argentina to 74% in the United States. At the time of diagnosis, patients' New York Heart Association functional class differed significantly between regions. At the time of last assessment, functional class had improved overall, and differences between regions had largely disappeared. A large proportion of patients did not undergo right heart catheterization for the diagnosis of PAH (Europe: 7%-21%; United States: 21%; Japan: 19%; Argentina: 51%). Variations in management included greater use of phosphodiesterase 5 inhibitors in the United States than in Europe and Japan and greater use of triple or greater combination therapy in Japan than in other regions. Results from this study, which includes a global aspect of PAH care, demonstrate that there are significant differences in PAH management between regions and low adherence to guidelines recommending right heart catheterization for the diagnosis of PAH.Keywords: pulmonary arterial hypertension, pulmonary arterial hypertension-specific therapies, diagnosis, clinical practice, guidelines. Pulmonary arterial hypertension (PAH; World Health Organization group I) is defined by a mean pulmonary artery pressure of ≥25 mmHg and increased pulmonary vascular resistance of >3 Wood units with a normal pulmonary capillary wedge pressure. 1,2 PAH is a progressive disease that can ultimately lead to right heart failure and death. 1,3 Despite advances in current therapies for PAH, there is still significant unmet medical need, especially in areas of the world where access to diagnostic and therapeutic options is limited. The mortality of patients with PAH remains high even in well-developed countries: 15% at 1 year and 32% at 3 years. 4 The therapeutic approach to PAH is evolving; multiple classes of agents are available, and physicians from both expert pulmonary hypertension (PH) centers and the community treat patients with PAH. 5,6 Data from various registries suggest that approaches to the diagnosis and treatment of PAH may differ between countries. [7][8][9][10][11] In this international study, we sought to assess differences in the diagnosis and management of patients with PAH across different countries and regions worldwide by conducting a large, physicianbased study using a quantitative online questionnaire. The objectives of the study were (1) to assess the diagnosis and management of PAH in different countries a...

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L) or LDL-C .300 mg/dL (8 mmol/L) under treatment with statins and/or 1 or both parents having clinically diagnosed FH. For the projection of the estimated prevalence of FH to the population of the Colombian Caribbean, the data of the adult population projections for the Colombian Caribbean of 2015 (general 10,442,134, adults 6,685,734) of the Departamento Administrativo Nacional de Estadisticas (DANE) were used. Results: If we assume that 1 of 5 patients with LDL-C .190 mg/dL (5 mmol/L) may have HF. The prevalence of patients with FH was 0.13% (87/64,667). The prevalence of adults with heterozygous FH (FHHe) was 0.13% (85/64,639). The prevalence of adults with homozygous HF (FHHo) was 0.0015% (1/64,639). Applying these estimates to the general population of Colombian Caribbean in 2015, the estimated number of cases of HF, FHHe and FHHo in the Caribbean Colombian could be approximately 13,574, 8,691 and 140, respectively. Conclusions: The estimated prevalence of FH, FHHe and FHHo in Colombian Caribbean was 1 of 769, 1 of 769 and 2 of 100,000 patients, respectively.

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